Contemporary survival outcomes of congenital systemic-to-pulmonary shunt in children with borderline pulmonary vascular resistant index compared to Eisenmenger syndrome.

Autor: Chaiwangyen N; Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand., Vijarnsorn C; Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand. cvijarnsorn@yahoo.com., Chungsomprasong P; Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand., Chanthong P; Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand., Kanjanauthai S; Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand., Thammasate P; Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand., Pacharapakornpong T; Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand., Bositthipichet D; Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand., Sengsim J; Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand., Soongswang J; Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand., Tocharoenchok T; Department of Surgery, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand., Nitiyarom E; Department of Surgery, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand., Tantiwongkosri K; Department of Surgery, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand., Subtaweesin T; Department of Surgery, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand., Durongpisitkul K; Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
Jazyk: angličtina
Zdroj: Scientific reports [Sci Rep] 2024 Jul 23; Vol. 14 (1), pp. 16867. Date of Electronic Publication: 2024 Jul 23.
DOI: 10.1038/s41598-024-67899-6
Abstrakt: Pulmonary arterial hypertension (PAH) associated with congenital heart disease (PAH-CHD) is a complication that occurs after unrepaired significant systemic-to-pulmonary shunt. Treatment options for PAH-CHD-predominantly left-to-right (L-R) shunt in children with borderline-high pulmonary vascular resistant index (PVRi) have been debated. We aimed to assess the treatment and survival of children with PAH-CHD-predominantly L-R shunt with borderline to high PVRi, using Eisenmenger syndrome (ES) for comparison. In 1995-2021, a total of 142 patients with ES and 192 children with PAH-CHD-predominantly L-R shunt were eligible for our analysis. The PVRi in ES patients was 26.7 ± 16.8 WU m 2 . Most patients (91%) received PAH-targeted therapy. Of the 192 children with PAH-CHD-predominantly L-R shunt, the baseline PVRi was 9.2 ± 5.8 WU m 2 . A total of 64 patients (33.3%) had borderline PVRi (4-8 WU m 2 ) and 98 patients (51%) had high PVRi (> 8 WU m 2 ). Most patients (88.5%) responded to acute pulmonary vasodilatory testing and underwent repair, with 158 undergoing defect closure and 12 having fenestrated closure. A treat-and-repair strategy was used in 33 children (17.1%). The 10- and 15-year survival rates for patients with ES were 79.3% and 72.4%, respectively, which was significantly inferior to children with borderline PVRi [97.3% and 87.8% (p = 0.02)]; and high PVRi [91.6% and 89.5% (p = 0.06)], respectively. The survival rate of children receiving treat-and-repair was slightly higher than that of ES (p = 0.16). The independent mortality risk in children with PAH-CHD-predominantly L-R shunt was persistent PAH following the defect correction (adjusted hazard ratio 5.8, 95% CI 1.7-19.9, p = 0.005).Trial registration: TCTR20200420004.
(© 2024. The Author(s).)
Databáze: MEDLINE
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